Ataxia

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  • See also

    Acute Poisoning 
    Head injury 
    Hypoglycaemia guideline 
    Metabolic disorders

    Key Points

    1. Ataxia results from uncoordinated muscle movements that cause poor balance, a staggered gait, difficulty sitting, unsteady and/or clumsy movements.
    2. Acute post infectious cerebellar ataxia is the most common cause and a benign and self-limited condition.
    3. Assessment focuses on excluding serious and treatable causes including central nervous system (CNS) infection or inflammation, stroke, toxin ingestion and mass lesions.
    4. Some conditions present with an unsteady gait due to weakness (pseudoataxia). A frightened or very unwell child may also appear ataxic.

    Background

    Classification & causes

    Acute ( <72 hours duration, previously well child)

    Episodic (recurrent ataxia)

    Chronic

    Post infectious*                                               
    Toxins*
    Tumours*
    Trauma
    Metabolic
    Infections
    Vascular – may require urgent lysis see Stroke
    Immune (eg. ADEM)
    Conversion disorder 

    Toxin ingestion  
    Basilar artery migraine
    Seizure disorder
    Metabolic 

    Brain tumours
    Hydrocephalus
    Metabolic
    Nutritional
    Congenital malformations
    Hereditary ataxias

    *Most common causes

    See Common Causes of Ataxia for further information.

    Assessment

    Red flag features in Red

    Any child with a red flag feature should not be considered to have acute cerebellar ataxia until further investigated.

    • Altered conscious state
    • Focal neurology
    • Signs of raised intracranial pressure
    • Meningism
    • Posterior column loss
    • Weakness
    • Reduced reflexes

    History

    • Time course (acute < 72hours)
    • Antecedents
      • Recent viral illness
      • Rash
      • Head trauma
      • Labyrinthitis (primary or secondary to otitis media)
    • Cerebellar signs
    • Diplopia
    • Difficulty sitting
    • Slurred speech 
    • Vomiting
    • Other neurological symptoms
      • Headache
      • Behavioural change
      • Photophobia
      • Vertigo
    • Family History
      • Metabolic disease
      • Hereditary ataxia
      • Migraine
      • Seizure disorder
    • Drugs/toxins or environmental exposures (heavy metals, gases, solvents)

    Examination

    • Conscious state, orientation.
    • Thorough neurological examination is mandatory.
    • Exclude signs of infection (fever, meningism).

    Features suggestive of Acute Cerebellar Ataxia

    • Cerebellar signs
    • Gait abnormalities (staggering and/or wide-based)
    • A negative Romberg test
    • Preservation of vibration and joint position sense
    • Absence of red flag features that suggest a more serious cause

    Management

    Investigations

    If there is a clear history of acute cerebellar ataxia with preceding viral prodrome, then no investigations may be necessary.
    If ataxia does not resolve in the expected time course or another cause is suspected, consider: 

    Imaging

    MRI (or CT if unavailable) to identify space occupying lesions, trauma or haemorrhage

    Bloods

    Blood gas (electrolytes, blood glucose)
    Anticonvulsant level, ethanol/ethylene glycol
    Full blood examination
    Liver function test

    Other

    Metabolic screening
    Lumbar puncture after neuroimaging
    EEG
    Toxicology

    Treatment

    • Children with acute cerebellar ataxia due to a viral illness can be discharged following senior doctor review and when close follow-up arrangements.
    • Children who have unexplained ataxia or ataxia from non-viral causes require admission for investigation.

    Consider consultation with local paediatric team when: 

    Child presents with an ataxia of unclear aetiology.

    Consider transfer when:

    • Localising signs
    • Raised ICP
    • History not typical of acute cerebellar ataxia or aetiology is unclear
    • Child requiring care beyond the comfort level of the hospital.   

    For emergency advice and paediatric or neonatal ICU transfers, see Retrieval Services.

    Consider discharge when:

    The cause of ataxia is clear and the child is well.

    Last updated December 2019

  • Reference List

    1. Ataxia. DeLong GR. Dershewitz RA, eds. Ambulatory Pediatric Care. 2nd 1998:594-596 Lippincott Philadelphia, Pa
    2. Ataxia: From the Benign to the Ominous. Dunn DW, Patel H. Contemporary Pediatrics. 1991;8:82-96
    3. Whelan, H.T., Verma, S., Guo, Y., Thabet, F., Bozarth, X., Nwosu, M., Katyayan, A., Parachuri, V., Spangler, K., Ruggeri, B.E. and Srivatsal, S., 2013. Evaluation of the child with acute ataxia: a systematic review. Pediatric neurology, 49(1), pp.15-24.